Positive HCM Outlook for Gene + Kids

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A study at the University of Amsterdam recently published in the Journal of Pediatrics found that most kids carrying a gene for HCM will not go on to develop HCM during their childhood.  

The same study also found that gene positive children without overt signs of the disease are at relatively low risk for cardiac events.

The study included 119 children, positive for at least one HCM gene, with a median age of 12.1 years.  8 of these children (6.7%) received a HCM diagnosis within the time span of the study [which varied from 3.1 to 10.7 years].  1 of the 8 diagnosed children suffered a cardiac event which necessitated implantation of an implantable cardioverter defibrillator or ICD.

The study did caution, however, that because severe hypertrophy and cardiac events may develop, it is important to refine risk stratification and long term follow up procedures for gene positive kids.

RESET-HCM: Rethinking Exercise for HCM Patients – Interview with Dr. Sara Saberi and Dr. Sharlene Day

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Editor’s note: This is our first interview feature on HCMBeat.  In the future, we hope to feature more interviews with other HCM researchers who have published articles of interest to the HCM community.  

By now, you have probably already heard the buzz about RESET- HCM – a study about the effects of exercise on HCM patients conducted by Dr. Sara Saberi and Dr. Sharlene Day at the Hypertrophic Cardiomyopathy Clinic of the University of Michigan’s Frankel Cardiovascular Center in collaboration with Dr. Matthew Wheeler and Dr. Euan Ashley of Stanford’s HCM Center. The findings were presented at the American College of Cardiology Conference on March 17, 2017 held in Washington D.C. and were the subject of this feature on HCMBeat. 

Recently, Cynthia Waldman of HCMBeat had the opportunity to sit down with Drs. Saberi and Day for a detailed conversation (over Skype) about the study.  What follows is a transcript of their conversation (which has been edited for readability).

Continue reading “RESET-HCM: Rethinking Exercise for HCM Patients – Interview with Dr. Sara Saberi and Dr. Sharlene Day” →

New Educational Resource for HCM Patients

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NOTE:  As of July, 2018 the website and app have been updated to include even more information for HCM patients. Check it out.

Have you heard that there is a new online educational resource about HCM? Check out HCM Care.com, an informational website about HCM developed by MyoKardia in partnership with Duke Clinical Research Institute.

This website features general information about HCM including diagnosis, testing, treatment, lifestyle, genetics and family screening.  It is also available as a FREE downloadable app for both Apple and Android operating systems.

Click here to find on iTunes and Google Play.
HCMCare features video clips from the following physicians:

  • Dr. James Daubert from Duke University Medical Center
  • Dr. Milind Desai from Cleveland Clinic
  • Dr. Carolyn Ho from Brigham and Women’s Hospital
  • Dr. Martin Maron from Tufts Medical Center
  • Dr. Andrew Wang from Duke University Medical Center

Be sure to check out HCMCare, as well as many other helpful resources which are listed on HCMBeat’s Resources page.

Exercise Helps HCM Patients – Results from RESET-HCM Study

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Results from a recent study conducted at the University of Michigan and Stanford show that patients who participated in a moderate-intensity cardiovascular exercise program showed a small, but statistically significant increase (6%) in exercise capacity over those who did not participate in the program.  Of note, no adverse events were reported in any of the 136 adults who participated in the RESET-HCM study over its four-month duration. These results were announced over the weekend at the American College of Cardiology meeting in Washington, D.C.

A companion editorial noted that this study is important for establishing the positive impact of exercise on HCM patients. Now, the need is for future research to establish safe exercise guidelines for HCM patients.  Many remain reluctant to exercise due to fear of suffering an adverse event during exercise.

Here is a video interview with one of the authors of the study, Dr. Sara Saberi, discussing the findings at ACC.

Stay tuned to HCMBeat for more about this important work which will hopefully lead to improved quality of life for HCM patients.

Study Shows MRIs Safe for Pacemaker & ICD Patients

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The long awaited results from the Magna-Safe study were published today in the New England Journal of Medicine, and it was good news for pacer and implantable cardioverter–defibrillator (ICD) patients! When proper precautions have been taken, MRIs were found to be safe in patients implanted with certain older pacemakers or ICDs.

In order to study the safety of scanning these patients, doctors set up a prospective registry for patients undergoing MRI in scanners with a magnetic field strength of 1.5 tesla who were scanned despite having a non-MR conditional device (i.e. a device which had not been deemed safe for MRI scanning by the FDA).

MRIs were performed on 1000 patients with pacemakers and 500 patients with ICDs and the results of the study showed that as long as appropriate patient selection and safety protocols were followed, patients had no problems with the MRI scans.

This is indeed good news for those of us with older devices who may be in need of MRI scans. To read about my own experiences and investigation of this issue, read my previous blog entry YES WE SCAN! ICDs and MRIs.

Apical Aneurysm is Risk Factor for HCM Patients

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A recent retrospective study of patients at Minneapolis Heart Institute and Tufts Medical Center published in the Journal of the American College of Cardiology found that HCM patients who also had left ventricular apical aneurysms were at increased risk of sudden cardiac death and stroke.  However, with increased surveillance and appropriate treatment, including the implantation of a implantable defibrillator, radiofrequency ablation and/or anti-coagulation, as appropriate, the authors suggest that the increased risk can be neutralized.

A summary of this article can be found here.

HCMA Founder Has Successful Heart Transplant

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Lisa Salberg, Founder and CEO of the Hypertrophic Cardiomyopathy Association, underwent a successful heart transplant last week at New Jersey’s Newark Beth Israel Hospital. Her  experience is featured in this article published today on NorthJersey.com  (The Bergen Record).

Lisa’s future plans include continuing her many advocacy efforts on behalf of HCM patients, as well as running in a 5K race.  We have a feeling that Lisa will be competing in Track & Field events at the Transplant Games of America before you know it!

EDITED TO ADD STORIES ABOUT LISA HOLDING HER OLD HEART IN HER HANDS ON VALENTINE’S DAY IN ORDER TO EDUCATE OTHERS ABOUT HCM.

Inside Edition (with video)

News 12 New Jersey (with video)

ABC News

BBC Radio Interview

Insurance Discrimination in Sudden Death Genetic Heart Conditions

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A recent study published in the American Heart Association’s Circulation found that a majority of individuals who have a Sudden Arrhythmia Death Syndrome (SADS) diagnosis and/or relatives applying for various types of insurance (such as health, life, travel, and disability) were denied coverage.  The preexisting SADS diagnosis was the predominant reason given for denial.

Though HCM does not generally fall under the SADS umbrella, these findings are equally concerning for HCM families.

SADS conditions generally include:

For more information on these conditions, visit the SADS Foundation.

 

 

Irish Researchers Investigate Drug for HCM

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EpiCor Therapeutics, a Irish biotech start-up, is investigating whether 5-azacytidine, a drug previously used to treat bone marrow disorders, may reverse the hypertrophy of HCM.  According to the researchers, the drug targets the mechanisms that drive the abnormal thickening of the heart muscle by inhibiting DNA methylation, thereby reducing the growth of cardiac cells.

EpiCor’s work with 5-azacytidine was declared the overall winner of the 2016 Venture Launch Accelerator Programme at Ireland’s University College Dublin where it won the 2016 Start-Up of the Year Award.

So far, EpiCor has gotten approximately €550,000 ($690,000 U.S.) in capitalization from Enterprise Ireland’s Commercialization Fund. The next step for the drug would be to obtain regulatory approval and then, clinical trials.

Could St. Jude ICDs be a Target for Hackers? FDA Issues Safety Advisory

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The U.S. Food and Drug Administration today issued a safety advisory regarding St. Jude Medical implantable cardiac devices used in conjunction with St. Jude’s proprietary Merlin @home Transmitter.

According to the advisory, these devices could potentially be vulnerable to hacking. However, only a highly skilled hacker would be sophisticated enough to exploit the vulnerability.  Such unwarranted interference could conceivably cause premature battery depletion or unnecessary shocks.

A software patch has been developed for the Merlin @home monitor designed to address the issue and to reduce the risk of hacking.  The update is now available and will be applied automatically to the Merlin monitor.

***Patients only need to make sure their Merlin@home Transmitter remains plugged in and connected in order to receive the software patch.***

Short-selling firm Muddy Waters first went public with this information in August, believing that it might cause a pending $25 billion acquisition of St. Jude Medical by Abbott Laboratories to fall apart. However, the deal closed last week despite the issue.

See also:

This article on Medscape

This article in the Minneapolis Star Tribune.

This article on Huffington Post.

This article on CNBC.